Chordomas are extremely rare tumours with undefined systemic treatment options in the advanced, metastatic setting. Pemetrexed has been proposed as a possible agent in this setting. We present a case of a patient who was initially managed curatively for a sacral coccygeal chordoma that recurred with liver metastatic disease nearly four years later. Palliative imatinib had to be discontinued due to skin toxicity. Based on limited case-based evidence, the patient was commenced on high-dose single-agent pemetrexed, a folate antimetabolite form of chemotherapy commonly used alongside other agents in the treatment of some lung cancers. Treatment dose could be escalated to 900mg/m2 within three cycles with minimal toxicity, and after a total of 12 cycles, imaging indicated a marked improvement in his metastatic disease burden. Metastatic chordomas have historically been difficult to control with systemic chemotherapy, and generation of evidence in rare cancers can be challenging. Pemetrexed may represent a systemic treatment option for patients with advanced chordomas.
Mohangee et al. (Mon,) studied this question.